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A case report of rare location of ganglioglioma

  • Vikas SharmaEmail author
  • S. Bhaskar
  • Sumit Ramdas Hire
  • Arvind Ahuja
Open Access
Case report
  • 82 Downloads

Abstract

Background

Gangliogliomas are rare tumors of the central nervous system. They can occur anywhere in the central nervous system but are most commonly located in the temporal lobe and are mainly found in children. Anaplastic ganglioglioma can result from either de novo or transformation of a pre-existing lesion.

Case presentation

We report a case of de novo anaplastic ganglioglioma in the parieto occipital region, which is a rare location. A 34-year-old lady presented with features of raised intracranial pressure (ICP) with right side hemiparesis. Contrast-enhanced magnetic resonance imaging (CEMRI) of the brain showed well-defined intense heterogenously enhancing solid cystic mass lesion 5.3 × 5.2 cm in the left parieto occipital region with mass effect and midline shift. Intraoperatively, a cystic mass lesion with reddish brown nodule was seen in the left occipital lobe. Complete tumor excision was done. Microscopic and IHC examination was suggestive of anaplastic ganglioglioma. The post-operative period was uneventful. The patient received 60-Gy radiotherapy with temozolamide as adjuvant therapy, and repeat imaging showed no tumor recurrence.

Conclusion

Anaplastic gangliogliomas are rare tumors with parieto occipital as rare location.

Keywords

Ganglioglioma Tumor Children Radiotherapy 

Abbreviations

CEMRI

Contrast-enhanced magnetic resonance imaging

ICP

Intracranial pressure

IHC

Immunohistochemistry

PAs

Pilocytic astrocytomas

PXAs

Pleomorphic xanthoastrocytomas

Background

Gangliogliomas are rare tumors, and they account for 1% (reported range of 0.4 to 7%) of all central nervous system neoplasms [1]. They are commonly seen in children and young adults with males affected more commonly than females [1]. These tumors occur throughout the CNS, but the temporal lobe is the commonest site [2]. The most common presentation is seizures. They are generally of WHO grades 1 and 2, and grade 3 (anaplastic ganglioglioma) is very rare. In 5% of cases, gangliogliomas show aggressive behavior and anaplastic histopathologic features characteristic of WHO grade 3 tumors [3]. These tumors can occur de novo or as a result of malignant transformation of a pre-existing lesion. We report here a rare case of de novo occipital anaplastic ganglioglioma. On searching the English literature, a total of 41 cases of de novo anaplastic ganglioglioma have been reported with only 4 cases in parieto occipital location.

The details of the previously reported cases have been summarized in Table 1.
Table 1

Previously published cases

S no

Authors/year

Age/sex

Location

Resection

Follow-up

RT

CT

1

Hall et al. 1986 [4]

6 months/F

Right frontal

Not mentioned

20 months

No

2

Lang et al. [4]

3

Hirose et al. 1992 [5]

12 years/F

Brain stem

Not mentioned

Not mentioned

Yes

Yes

5

Wolf et al. 1994 [6]

6

 

7

Prayson et al. 1995 [4]

8

Campos et al. 1994 [4]

10 years/M

Left temporal

6 months

9

Hakim et al. 1997 [4]

47 years/M

Frontal

Total

14 months

SRS

Yes

10

Nakajima et al. 1998 [4]

7 years/F

Right parieto occipital region

Subtotal

15 months

Yes

Yes

11

Araki et al. 1999 [4]

53 years/F

Frontal

Total

26 months

Yes

Yes

12

Danjoux et al. 2001 [4]

26 years/M

Temporal

2 months

Yes

13

Suzuki et al. 2002 [4]

59 years/M

Temporal

14

Nair et al. 2004 [4]

60 years/M

Right parietal

15

Lyken et al. 2004 [4]

11 years

Yes

Yes

16

 

Yes

Yes

17

Kang et al. 2007 [4]

45 years/M

Left frontal

Subtotal resection

35 months

Yes

Yes

18

Majores et al. 2008 [7]

45 years/M

Left temporo mesial

Total

11 months

Yes

Yes

19

 

M

Yes

Yes

20

 

M

Yes

Yes

21

 

M

Yes

Yes

22

 

F

Yes

Yes

23

Karremann et al. 2009 [4]

2 years/M

Left fronto basal

Total

31 months

No

No

24

 

3 years/M

Right frontal

Total

54 months

No

Yes

25

 

10 years/F

Right frontotemporoparietal

Total

42 months

Yes

Yes

26

 

10 years/M

Right parieto occipital

Partial

6 months

Yes

Yes

27

 

14 years/M

Pons and fourth ventricle

Subtotal

8 months

Yes

No

28

 

16 years/F

Right temporo mesial

Total

25 months

Yes

No

29

Kawataki et al. 2010 [4]

34 years/M

Left medial temporal

Subtotal

6 months

Yes

Yes

30

A.E. Rojas et al. 2013 [8]

22 years/M

Left frontotemporal lobes

11 months

Yes

31

 

24 years/F

Left frontotemporal lobes

3 months

Yes

32

 

25 years/M

Left frontotemporal lobes

6 months

Yes

33

 

34 years/M

Left frontal

9 months

Yes

34

 

9 years/M

Posterior fossa

6 months

Yes

35

 

9 years/M

B/L basal ganglia

2 months

Yes

Yes

36

 

37 years/M

Left frontotemporal region

2 months

Yes

37

Lucas et al. 2015 [9]

12 years/M

Left temporoparietal

Subtotal

23 months

Yes

Yes

38

 

11 years/M

Right temporal

Subtotal

22 months

Yes

Yes

39

Pikis et al. 2017 [10]

34 years/F

Left frontal

Subtotal

12 months

Yes

Yes

40

Lundar et al. 2018 [11]

15 years/M

Supratentorial

Total

14 years

41

 

19 years/M

Supratentorial

Total

12 months

42

Present study

34 years/F

Left parieto occipital

Total

26 months

Yes

Yes

Case presentation

A 34-year-old female presented with complaints of on/off headache with vomiting for 1 year, which aggrevated for the last 5 days. Systemic examination was within normal limits. GCS was E4V5M6, pupils were bilateral normal size and reacting, on fundus examination-bilateral papilledema was present. On the motor system examination, right side hemiparesis was present (motor power 4/5). Contrast-enhanced magnetic resonance imaging (CEMRI) of the brain showed well-defined intense heterogenously enhancing solid cystic mass lesion 5.3 × 5.2 cm in the left parieto occipital region. Solid component was isointense on T2/FLAIR images with peripheral cystic areas and isointense on T1W images. The cystic component was iso- to hyperintense on T2/FLAIR images and hypointense on T1W images. The lesion showed mass effect in form of compression and displacement of the occipital horn of the lateral ventricle and compression of the brain stem with midline shift and brain edema. These features were suggestive of hemangioblastoma/pilocytic astrocytoma (Fig. 1).
Fig. 1

CEMRI of the brain shows a well-defined solid cystic lesion in the left parieto occipital region. Solid component isointense and cystic component hypointense on T1 axial images (a). Solid component isointense and cystic component iso- to hyperintense on T2 axial images. Solid component shows intense enhancement on T1 contrast coronal and sagittal images (c, d), Solid component isointense and cystic component iso- to hyperintense on T2 axial images (b)

Left parieto occipital craniotomy was done which showed a cystic mass lesion with reddish brown solid nodule in the occipital region. Cyst was tapped followed by nodule excision. The nodule was soft to firm in consistency and was mild to moderately vascular. Tumor was extending into the occipital horn and midline up to the falx; however, no attachment to the falx was seen gross total tumor excision was done.

Histopathology

Microscopic examination showed cellular glial tumor composed of malignant astrocytic cells and scattered ganglion cells. Astrocytic cells showed moderate nuclear pleomorphism with coarse chromatin with few mitosis. Immunohistochemistry (IHC) analysis showed focal positivity (ganglion cells) for CD56 and was negative for CD34, P53, IDH1, and synaptophysin. MIB1 index was 8% (Figs. 2 and 3). These features were suggestive of anaplastic ganglioglioma, WHO grade 3.
Fig. 2

Photomicrograph showing a branching capillaries and eosinophilic granular bodies seen in a fibrillary glial background. H&E, × 100. b Ganglion cells with vesicular chromatin, prominent nucleoli, and moderate to abundant amount of eosinophilic cytoplasm. H&E, × 400. c Photomicrograph showing a mitotic figure. H&E, × 100. d Cellular glial tumor composed of malignant astrocytic cells with scattered ganglion cells and marked vascular proliferation. H&E, × 100

Fig. 3

HC a ganglion cells showing cytoplasmic positivity for CD 56. b Ki67/MIB1positivity

The immediate post-operative period was uneventful. The patient made gradual recovery over the and received 60-Gy radiotherapy with temozolamide as adjuvant therapy. Post-operative CEMRI of the brain showed craniotomy defect with gliosis in the left parieto occipital region with no contrast enhancement (Fig. 4). CEMRI of the brain was done 14 and 24 months after surgery which showed post radiotherapy changes with gliosis with no recurrent lesion (Fig. 5). The patient is asymptomatic 26 months after surgery.
Fig. 4

Post-op CEMRI of the brain shows post-operative changes with no contrast enhancement. T1 and T2 axial (a, b) and T1 with contrast coronal and sagittal (c, d)

Fig. 5

CEMRI brain T1 contrast image done after 14 months of surgery showing post radiotherapy changes with no enhancement

Discussion

Ganglioglioma is an infrequent tumor of the central nervous system, initially characterized by Perkins in 1926. The age of presentation of these tumors varies from 2 months to 70 years. The majority of gangliogliomas occur in the temporal lobe (> 70%) [7]. The most common presenting signs and symptoms are seizures (temporal lobe and other supratentorial locations), followed by headache, dizziness, ataxia (posterior fossa), and progressive weakness (spinal cord). The typical of ganglioglioma is a benign, calcified tumor in the temporal lobe of a child with seizures [3]. On CT, the picture is of a circumscribed solid mass or cyst with a mural nodule. On MR imaging, gangliogliomas are isointense to hypointense on T1-weighted images, are hyperintense and heterogeneous on T2-weighted images, and can contain solid, cystic, and calcified components. Enhancement after administration of gadolinium has also been found to vary from no enhancement to marked, heterogeneous enhancement [2, 12].

Histopathologically, gangliogliomas are benign, well-differentiated neuroepithelial tumors. The typical feature is a combination of neuronal and glial cell elements, which may exhibit marked heterogeneity [13]. On IHC, the glial component is positive for GFAP, S-100 protein, and vimentin, whereas the neuronal component is reactive for synaptophysin, MAP 2, NeuN, and neurofilaments [14, 15]. Histopathologic differential diagnoses comprise both high-grade and low-grade neoplasms, such as diffuse astrocytomas, oligodendrogliomas, dysembryoplastic neuroepithelial tumors, pilocytic astrocytomas (PAs), and pleomorphic xanthoastrocytomas (PXAs) [7]. Mitotic figures in ganglioglioma are rare, and MIB index varies from 1.1 to 2.7%. CD 34 is present 70–80% in the neuronal component of ganglioglioma but is less common in anaplastic variants [15]. IDH1 and P53 should be done to rule out diffuse glioma.

Anaplastic gangliogliomas typically demonstrate malignant transformation of the glial component with hypercellularity, vascular proliferation, and necrosis high mitotic labeling indices (Ki-67) [16, 17, 18]. Anaplastic transformation is more common in the pediatric population and has been associated with previous subtotal tumor resection and radiotherapy [19]. Gross total resection should be attempted whenever possible with the preservation of neurological function. Some studies have advocated the role of gross total resection along with adjuvant radiochemotherapy in achieving good survival rates [20, 21].

Conclusion

Anaplastic gangliogliomas are rare tumors with parieto occipital as rare location. Imaging may vary, and histology along with IHC is needed for the diagnosis. Gross total resection along with adjuvant therapy improves outcome.

Notes

Acknowledgements

Not applicable.

Authors’ contributions

VS and SB contributed to the data collection and writing of the manuscript. AH contributed to the histopathology and IHC analysis part. VS and SRH contributed to the cross-checking and editing of the manuscript. All authors read and approved the final manuscript.

Funding

There is no external funding for the publication of this article.

Ethics approval and consent to participate

As this is a case report, ethical clearance is not required.

Consent for publication

Consent for publication was taken from the patient prior to reporting of the case.

Competing interests

The authors declare that they have no competing interests.

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Authors and Affiliations

  1. 1.Department of NeurosurgeryBombay HospitalMumbaiIndia
  2. 2.Department of NeurosurgeryPGIMER & Dr RML HospitalNew DelhiIndia
  3. 3.Department of PathologyPGIMER & Dr RML HospitalNew DelhiIndia

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